Use of Regional Anaesthesia in a Patient With Acute Porphyria

McNeill, M. J.; Bennet, A.

doi:10.1097/00132582-199007000-00069

Cited by 3 publications

(5 citation statements)

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“…To set against these problems, because of the absence of liver disease in acute porphyrias, patients typically have normal coagulation and no portal hypertension. They may therefore be suitable for combined general anesthesia and epidural blockade with procaine or bupivacaine may be used 36. thus providing the benefit of postoperative analgesia with little respiratory depression.…”

Section: Autosomal Dominant Acute Porphyriasmentioning

confidence: 99%

Liver transplantation for porphyria: Who, when, and how?

et al. 2007

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Porphyrias are a heterogenous group of diseases that may result in disabling or life threatening neurovisceral symptoms and/or cutaneous photosensitivity. In acute intermittent porphyria, the clinical features, particularly neurological symptoms, may be life-threatening and disabling. Conventional treatment with human hemin, though effective in reducing symptoms, does not reverse neuropathy when structural nerve damage has occurred and may cause intense phlebitis. Liver transplantation (LT) may be considered as treatment for those with repeated life-threatening acute attacks resulting in poor quality of life, requirement of ventilatory support, and progressive loss of venous access due to hemin infusion. Patients with variegate porphyria (VP) present after puberty with neurovisceral symptoms and skin manifestations. LT resolved VP in the 1 patient reported in the literature. Aminolaevulinic acid dehydratase deficient porphyria is a rare autosomal recessive disorder and a child who presented with failure to thrive and required transfusions and parenteral nutrition did not improve with LT. In erythropoietic protoporphyria (EPP), there is excessive production of protoporphyrin in the bone marrow. Protoporphyrin is hepatotoxic and pigment loading of hepatocytes and bile canalicular sludging may result in progressive cholestasis and cirrhosis. LT is beneficial for such patients with end-stage liver disease. Perioperative management includes use of filters on operative lights to prevent skin burns and intestinal perforation. Other concerns include development of neuropathy, biliary complications, and recurrent liver disease. This review addresses the rationale, patient selection, evaluation, management issues, and technique of performing LT in various types of porphyria.

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Section: Autosomal Dominant Acute Porphyriasmentioning

confidence: 99%

Liver transplantation for porphyria: Who, when, and how?

et al. 2007

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“…Parikh and Moore examined various agents in a rat model which suggested that procaine and bupivacaine are safe agents, but that lidocaine should be avoided [16]. There is no evidence that the use of bupivacaine in a regional anesthetic technique precipitates an attack of acute intermittent porphyria [6]. Epidural administration of a mixture of fentanyl and bupivacaine was effective for postoperative pain relief in our patient, without any complications.…”

Section: Discussionmentioning

confidence: 74%

“…However, the safe use of regional anesthesia has been reported in porphyria [6,7]. Carp and Clark have suggested that the use of regional anesthesia is not contraindicated as long as the possibility of autonomic dysfunction is considered [15].…”

Section: Discussionmentioning

confidence: 99%

“…Although a variety of drugs have been implicated in precipitating an acute attack [5], it is difficult to predict which drugs will be porphyrogenic. There have been several reports about the anesthetic management of patients with AIP [4,6,7], but the number of case reports about HCP is small [8,9]. We report the anesthetic management of a patient with HCP.…”

Section: Introductionmentioning

confidence: 96%

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Safety of isoflurane and epidural anesthesia in a patient with hereditary coproporphyria

et al. 1996

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“…paracervical block) in porphyria is ill defi ned. McNeill and Bennet [5] suggest that regional anaesthesia should be avoided when neurological symptoms may occur after operation, for medico-legal reasons, but is by no means contraindicated. Lignocaine has theoretically shown to be porphyrinogenic in animal models but wide experience with this agent has not shown any signifi cant problems when used as a local anaesthetic or when used intravenously for control of arrhythmias [6] .…”

Section: Discussionmentioning

confidence: 99%

Medical Termination of Pregnancy in Acute Intermittent Porphyria

Sahu¹,

Rajaram²,

Saxena

et al. 2006

Gynecol Obstet Invest

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Acute intermittent porphyria is a rare autosomal dominant disease characterized by acute attacks of neuropsychiatric and neurovisceral dysfunction. In pregnancy, exacerbation of attacks in the form of seizures and acute abdomen occur due to hormonal changes. We report the case of a young woman presenting at 8 weeks of gestation requesting pregnancy termination. The patient suffered several episodes of acute abdomen and seizures in a prior pregnancy before the diagnosis was clinched. This case is reported because of its rarity and the limited obstetric experience of this disorder. Proper management depends on avoidance of precipitating factors. Anaesthetic drug safety in porphyria is reviewed and the choice of regional anaesthesia discussed

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Use of Regional Anaesthesia in a Patient With Acute Porphyria

Cited by 3 publications

References 0 publications

Liver transplantation for porphyria: Who, when, and how?

Liver transplantation for porphyria: Who, when, and how?

Safety of isoflurane and epidural anesthesia in a patient with hereditary coproporphyria

Medical Termination of Pregnancy in Acute Intermittent Porphyria

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